Protein-Losing Enteropathy
A serious complication of Fontan circulation characterized by excessive protein loss through the gastrointestinal tract, leading to hypoproteinemia and edema.
Full Definition
Protein-losing enteropathy (PLE) is a devastating late complication that occurs in approximately 5-10% of patients with Fontan circulation, typically manifesting years after the initial surgery. The condition is characterized by excessive loss of plasma proteins through the intestinal tract, resulting in hypoproteinemia, hypoalbuminemia, and peripheral edema. The pathophysiology involves elevated systemic venous pressure, lymphatic dysfunction, and chronic inflammation leading to increased intestinal permeability. PLE is associated with poor prognosis and may require aggressive medical management including dietary modifications, albumin supplementation, diuretics, and in severe cases, heart transplantation. The condition can also occur in other forms of congenital heart disease with chronically elevated right heart pressures.
Usage
Usage note: Often abbreviated as PLE in clinical contexts involving Fontan patients.
In Context
- "The adolescent Fontan patient developed protein-losing enteropathy with severe hypoalbuminemia and ascites." — Gastroenterology consultation
- "Management of protein-losing enteropathy included high-protein diet, diuretics, and consideration for cardiac transplantation." — Multidisciplinary care plan